Glycogen‐binding protein STBD1: Molecule and role in pathophysiology

Abstract

Starch‐binding domain‐containing protein 1 (STBD1) is a glycogen‐binding protein discovered in skeletal muscle gene differential expression that is pivotal to cellular energy metabolism. Recent studies have indicated that STBD1 is involved in many physiological processes, such as glycophagy, glycogen accumulation, and lipid droplet formation. Moreover, dysregulation of STBD1 causes multiple diseases, including cardiovascular disease, metabolic disease, and even cancer. Deletions and/or mutations in STBD1 promote tumorigenesis. Therefore, STBD1 has garnered considerable interest in the pathology community. In this review, we first summarized the current understanding of STBD1, including its structure, subcellular localization, tissue distribution, and biological functions. Next, we examined the roles and molecular mechanisms of STBD1 in related diseases. Based on available research, we discussed the novel function and future of STBD1, including its potential application as a therapeutic target in glycogen‐related diseases. Given the significance of STBD1 in energy metabolism, an in‐depth understanding of the protein is crucial for understanding physiological processes and developing therapeutic strategies for related diseases.

From Acid Alpha-Glucosidase Deficiency to Autophagy: Understanding the Bases of POMPE Disease

“…This process has been proposed in PD based on the increased Stbd1 levels (starch binding domain 1) found in GAA-KO mice, which is the protein believed to transport glycogen inside the lysosome [30,31]. Moreover, recently, Stbd1 has been proposed as a potential target for therapy based on evidence of reduction in lysosomal glycogen accumulation in STBD1/GAA double knockout mice [31,32].…”